Calcinosis cutis | |
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Other names | Cutaneous calcification |
Micrograph of calcinosis cutis. The calcification is purple (bottom of image). H&E stain. | |
Specialty | Dermatology |
Calcinosis cutis is an uncommon condition marked by calcium buildup in the skin and subcutaneous tissues. Calcinosis cutis can range in intensity from little nodules in one area of the body to huge, crippling lesions affecting a vast portion of the body. [1] Five kinds of the condition are typically distinguished: calciphylaxis, idiopathic calcification, iatrogenic calcification, dystrophic calcification, and metastatic calcification. [2]
Tumors, inflammation, varicose veins, infections, connective tissue disease, hyperphosphatemia, and hypercalcemia can all lead to calcinosis. Systemic sclerosis is linked to calcineuris cutis. [3] Calcinosis is seen in Limited Cutaneous Systemic Sclerosis, also known as CREST syndrome (the "C" in CREST). [4]
Lesions might be more severe and widespread, or they can develop gradually and show no symptoms. The nodules may cause pain and hinder function in addition to having a variety of sizes and shapes. The underlying condition determines the localization of the lesions in dystrophic calcification. The elbows, fingers, knees, and forearms are the most often affected regions in people with systemic sclerosis. Elbows, knees, and regions of prior inflammatory lesions in dermatomyositis are affected by calcification. Lupus erythematosus affects the limbs, buttocks, area beneath lupus lesions, and periarticular areas. Periarticular lesions are found in metastatic calcification. In tumoral calcinosis, the lesions are found around joints, but in idiopathic calcification, the lesions are found on children's faces as subepidermal calcified nodules. In iatrogenic calcification, the calcification is found at venipuncture sites. [3]
Calcinosis may result from a variety of causes such as: [3]
Calcinosis cutis is associated with systemic sclerosis. [3]
Calcinosis cutis may be divided into the following types: [5] : 527–530
Dystrophic calcinosis cutis is the most prevalent kind of calcification on the skin. [2] The ectopic calcified mass usually consists of amorphous calcium phosphate and hydroxyapatite. [6] Dystrophic calcification is linked to a number of illnesses, such as infections, hereditary diseases, cutaneous neoplasms, and connective tissue diseases. [7] The clinical manifestation can be as minor as an accidental radiography imaging finding or as severe as subcutaneous nodules or plaques. [8]
Metastatic calcinosis cutis is the consequence of calcium salts precipitating in normal tissue due to an underlying abnormality in the metabolism of phosphate and/or calcium. [2] Metastatic calcification can result from any systemic condition raising serum calcium and/or phosphate levels. Chronic renal failure is the most frequent underlying cause. [9]
Iatrogenic calcinosis cutis is characterized by firm nodules in the subcutis or dermis, which are caused by calcium salts precipitating quickly in the skin. [9] This occurrence typically manifests as a warm, sensitive swelling at the site of venipuncture, [2] and it most frequently happens following the extravasation of intravenous calcium chloride, calcium gluconate, or phosphate-containing solutions. [10] Iatrogenic calcification, which manifests as soft yellow-white epidermal plaques, [2] has also been linked to calcium salt exposure via electroencephalography or electromyographic electrode compounds. [11]
Traumatic calcinosis cutis is a cutaneous condition characterized by calcification of the skin resulting from the deposition of calcium and phosphorus often resulting from occupational exposure, as in cases reported in oil-field workers and coal miners. [12] : 528
Skin calcification that is not linked to a systemic illness or an underlying tissue injury is referred to as idiopathic calcification. [2] Most often, the calcification is restricted to a single general location, yet there has been one case of calcinosis cutis that is exceptionally broad. [13]
Idiopathic scrotal calcinosis is a cutaneous condition characterized by calcification of the skin resulting from the deposition of calcium and phosphorus occurring on the scrotum. [14] : 528 However, the levels of calcium and phosphate in the blood are normal. [15] Idiopathic scrotal calcinosis typically affects young males, with an onset between adolescence and early adulthood. [15] The scrotal calcinosis appears, without any symptoms, as yellowish nodules that range in size from 1 mm to several centimeters. [16]
Subepidermal calcified nodule is characterized by calcification of the skin resulting from the deposition of calcium and phosphorus, occurring most frequently as one or a few skin lesions on the scalp or face of children. [17] : 528
Tumoral calcinosis is distinguished by the accumulation of calcific masses surrounding the main joints. It mainly affects teens who are otherwise in good health. Joint function may be hampered by the subcutaneous or intramuscular calcified deposits. [2]
Osteoma cutis is a cutaneous condition characterized by the presence of bone within the skin in the absence of a preexisting or associated lesion. [18] : 529 Osteoma cutis often manifests as solid, varying-sized, skin-colored subcutaneous nodules. [19]
Diltiazem, a calcium channel blocker, has been a mainstay of medical treatment for calcinosis cutis. [1] It is thought to work by modifying intracellular calcium levels, which reduces the capacity for the production and crystallization of calcium nidus. [20]
Colchicine is an antimicrotubule drug with anti-inflammatory properties that has been used for gouty arthritis treatment for a long time. [1] Calcinosis cutis inflammation brought on by a foreign body-like response aggravates the illness's symptoms. [21] Colchicine, therefore, has been used to treat calcinosis cutis, albeit with varying degrees of success. [1]
Panniculitis is a group of diseases whose hallmark is inflammation of subcutaneous adipose tissue. Symptoms include tender skin nodules, and systemic signs such as weight loss and fatigue.
Dystrophic calcification (DC) is the calcification occurring in degenerated or necrotic tissue, as in hyalinized scars, degenerated foci in leiomyomas, and caseous nodules. This occurs as a reaction to tissue damage, including as a consequence of medical device implantation. Dystrophic calcification can occur even if the amount of calcium in the blood is not elevated, in contrast to metastatic calcification, which is a consequence of a systemic mineral imbalance, including hypercalcemia and/or hyperphosphatemia, that leads to calcium deposition in healthy tissues. In dystrophic calcification, basophilic calcium salt deposits aggregate, first in the mitochondria, then progressively throughout the cell. These calcifications are an indication of previous microscopic cell injury, occurring in areas of cell necrosis when activated phosphatases bind calcium ions to phospholipids in the membrane.
Calcinosis is the formation of calcium deposits in any soft tissue. It is a rare condition that has many different causes. These range from infection and injury to systemic diseases like kidney failure.
Sclerodactyly is a localized thickening and tightness of the skin of the fingers or toes that yields a characteristic claw-like appearance and spindle shape of the affected digits, and renders them immobile or of limited mobility. The thickened, discolored patches of skin are called morphea, and may involve connective tissue below the skin, as well as muscle and other tissues. Sclerodactyly is often preceded by months or even years by Raynaud's phenomenon when it is part of systemic scleroderma.
Angiokeratoma is a benign cutaneous lesion of capillaries, resulting in small marks of red to blue color and characterized by hyperkeratosis. Angiokeratoma corporis diffusum refers to Fabry's disease, but this is usually considered a distinct condition.
Granuloma annulare (GA) is a common, sometimes chronic skin condition which presents as reddish bumps on the skin arranged in a circle or ring. It can initially occur at any age, though two-thirds of patients are under 30 years old, and it is seen most often in children and young adults. Females are two times as likely to have it as males.
Leukemia cutis is the infiltration of neoplastic leukocytes or their precursors into the skin resulting in clinically identifiable cutaneous lesions. This condition may be contrasted with leukemids, which are skin lesions that occur with leukemia, but which are not related to leukemic cell infiltration. Leukemia cutis can occur in most forms of leukemia, including chronic myeloid leukemia, acute lymphoblastic leukemia, chronic lymphocytic leukemia, acute myeloid leukemia, and prolymphocytic leukemia.
Palisaded neutrophilic and granulomatous dermaititis (PNGS) is usually associated with a well-defined connective tissue disease, lupus erythematosus or rheumatoid arthritis most commonly, and often presents with eroded or ulcerated symmetrically distributed umbilicated papules or nodules on the elbows.
Infantile systemic hyalinosis is an allelic autosomal-recessive condition characterized by multiple skin nodules, hyaline deposition, gingival hypertrophy, osteolytic bone lesions and joint contractures.
Angiolipoleiomyoma is an acquired, solitary, asymptomatic acral nodule, characterized histologically by well-circumscribed subcutaneous tumors composed of smooth muscle cells, blood vessels, connective tissue, and fat.
Mucinoses are a group of cutaneous diseases caused by fibroblasts producing abnormally large amounts of acid mucopolysaccharides, usually hyaluronic acid.
Elephantiasis nostras is a disease that usually affects the lower legs or scrotum. Swelling is accompanied by rough nodules or wart-like plaques on the skin. If the disease is not treated, it eventually results in pain and immobility.
Cutaneous lymphoid hyperplasia refers to a groups of benign cutaneous disorders characterized by collections of lymphocytes, macrophages, and dendritic cells in the skin. Conditions included in this groups are:
Primary cutaneous marginal zone lymphomas represent a heterogeneous group of diseases characterized by solitary or multiple dermal or subcutaneous nodules. Lymphomas included in this group are:
Plasmacytosis is a condition in which there is an unusually large proportion of plasma cells in tissues, exudates, or blood. Plasmacytosis may be divided into two types—cutaneous and systemic—both of which have identical skin findings. Patients with plasmacytosis have been predominantly found to have lung infections whereas multiple myeloma is rarely found.
Idiopathic scrotal calcinosis is a cutaneous condition characterized by calcification of the skin resulting from the deposition of calcium and phosphorus occurring on the scrotum. However, the levels of calcium and phosphate in the blood are normal. Idiopathic scrotal calcinosis typically affects young males, with an onset between adolescence and early adulthood. The scrotal calcinosis appears, without any symptoms, as yellowish nodules that range in size from 1 mm to several centimeters.
Osteoma cutis is a cutaneous condition characterized by the presence of bone within the skin in the absence of a preexisting or associated lesion. Osteoma cutis often manifests as solid, varying-sized, skin-colored subcutaneous nodules.
A sebaceous adenoma, a type of adenoma, a cutaneous condition characterized by a slow-growing tumor usually presenting as a pink, flesh-coloured, or yellow papule or nodule.
Plate-like osteoma cutis is a congenital condition characterized by firm papules and nodules on the skin.